Abstract
Myelofibrosis, also called myeloid metaplasia, is a myeloproliferative disorder characterized by progressive bone marrow fibrosis, splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, anisopoikilocytosis, and teardrop poikilocytes. Most patients have moderate to severe anemia. Bone marrow in patients with myelofibrosis is usually hypocellular, with increased amounts of reticulin and collagen. Myelofibrosis may be primary (or idiopathic) or secondary, and usually affects adults, with only a few reports in children (Guermazi et al. 1999). Myelofi brosis is of unknown etiology, but association with an autoimmune diseases such as polyarteritis nodosa (Camos et al. 2003), ulcerative colitis (Arellano-Rodrigo et al. 2002), or primary biliary cirrhosis (Hernandez-Boluda et al. 2002) gives support to the hypothesis of an immune basis of some idiopathic myelofibrosis cases. The average life expectancy of patients after diagnosis is 2 to 3 years. Only 8% of patients with the disease survive from 6 to 8 years. The common imaging findings in patients with myelofibrosis are osteosclerosis, hepatosplenomegaly, and lymphadenopathies. In addition, extramedullary hematopoiesis may develop in multiple sites such as chest, abdomen, pelvis, and central nervous system, simulating malignant disease (Guermazi et al. 1999). The purpose of this chapter is to illustrate the wide range of radiological abnormalities in patients affected by this serious, progressive and fatal disease.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Abbitt PL, Teates CD (1989) The sonographic appearance of extramedullary hematopoiesis in the liver. J Clin Ultrasound 17:280–282
Alpdogan O, Budak-Alpdogan T, Bayik M, Akoglu T, Kodalli N, Gurmen N (1998) Magnetic resonance imaging in myelofibrosis. Blood 92:2995–2997
Amano Y, Onda M, Amano M, Kumazaki T (1997) Magnetic resonance imaging of myelofibrosis. STIR and gadoliniumenhanced MR images. Clin Imaging 21:264–268
Arellano-Rodrigo E, Esteve J, Gine E, Panes J, Cervantes F (2002) Idiopathic myelofi brosis associated with ulcerative colitis. Leuk Lymphoma 43:1481–1483
Aytaç S, Fitoz S, Akyar S, Atasoy C, Erekul S (1999) Focal intrahepatic extramedullary hematopoiesis: color Doppler US and CT fi ndings. Abdom Imaging 24:366–368
Bouroncle BA, Doan CA (1962) Myelofi brosis: clinical, hematologic and pathologic study of 110 patients. Am J Med Sci 243:697–715
Camos M, Arellano-Rodrigo E, Abello D, Muntanola A, Ferrer A, Grau JM, Cervantes F (2003) Idiopathic myelofi brosis associated with classic polyarteritis nodosa. Leuk Lymphoma 44:539–541
Chao PW, Farman J, Kapelner S (1986) CT features of presacral mass: an unusual focus of extramedullary hematopoiesis. J Comput Assist Tomogr 10:684–685
Coates GG, Eisenberg B, Dail DH (1994) Tc-99m sulfur colloid demonstration of diffuse pulmonary interstitial extra-medullary hematopoiesis in a patient with myelofibrosis. A case report and review of the literature. Clin Nucl Med 19:1079–1084
Cohn SL, Cohn RA, Chou P, Donaldson JS, Langman CB (1991) Infantile myelofi brosis with nephromegaly secondary to myeloid metaplasia. Clin Pediatr (Phila) 30:59–61
Connelly TJ, Abruzzo JL, Schwab RH (1982) Agnogenic myeloid metaplasia with polyarteritis. J Rheumatol 9 954–956
Cook G, Sharp RA (1994) Spinal cord compression due to extramedullary haemopoiesis in myelofibrosis. J Clin Pathol 47:464–465
Cufer T, Bracko M (2001) Myeloid metaplasia of the breast. Ann Oncol 12:267–270
De Klippel N, Dehou MF, Bourgain C, Schots R, De Keyser J, Ebinger G (1993) Progressive paraparesis due to thoracic extramedullary hematopoiesis in myelofibrosis. Case report. J Neurosurg 79:125–127
Diamond T, Smith A, Schnier R, Manoharan A (2002) Syndrome of myelofibrosis and osteosclerosis: a series of case reports and review of the literature. Bone 30: 498–501
Fernbach SK, Feinstein KA (1992) Extramedullary hematopoiesis in the kidneys in infant siblings with myelofibrosis. Pediatr Radiol 22:211–212
Gil-Fernandez JJ, Martinez-Chamorro C, Tomas JF (2001) The irreplaceable image: A giant hepatic mass of myeloid metaplasia in a patient without myelofibrosis. Haematologica 86:445
Glew RH, Haese WH, McIntyre PA (1973) Myeloid metaplasia with myelofibrosis. The clinical spectrum of extramedullary hematopoiesis and tumor formation. Johns Hopkins Med J 132:253–270
Goodman P, Kumar R, Alperin JB (1991) Diffuse intraabdominal lymphoma complicating idiopathic myelofibrosis: CT demonstration. AJR Am J Roentgenol 156:1189–1190
Gryspeerdt S, Oyen R, Van Hoe L, Baert AL, Boogaerts M (1995) Extramedullary hematopoiesis encasing the pelvicalyceal system: CT findings. Ann Hematol 71:53–56
Guermazi A, Miaux Y, Chiras J (1997) Imaging of spinal cord compression due to thoracic extramedullary haematopoiesis in myelofibrosis. Neuroradiology 39:733–736
Guermazi A, de Kerviler E, Cazals-Hatem D, Zagdanski AM, Frija J (1999) Imaging fi ndings in patients with myelofibrosis. Eur Radiol 9:1366–1375
Harris BK, Ross HA (1974) Hemarthrosis as the presenting manifestation of myeloproliferative disease. Arthritis Rheum 17:969–970
Hasselbalch H (1990) Idiopathic myelofi brosis: a clinical study of 80 patients. Am J Hematol 34:291–300
Heinicke MH, Zarrabi MH, Gorevic PD (1983) Arthritis due to synovial involvement by extramedullary haematopoiesis in myelofibrosis with myeloid metaplasia. Ann Rheum Dis 42:196–200
Hernandez-Boluda JC, Jimenez M, Rosinol L, Cervantes F (2002) Idiopathic myelofi brosis associated with primary biliary cirrhosis. Leuk Lymphoma 43:673–674
Holt SG, Field P, Carmichael P, Mehta A, Jarmulowicz M, Clarke D, Hilson A, Burns A (1995) Extramedullary haematopoeisis in the renal parenchyma as a cause of acute renal failure in myelofibrosis. Nephrol Dial Transplant 10: 1438–1440
Humphrey PA, Vollmer RT (1991) Extramedullary hematopoiesis in the prostate. Am J Surg Pathol 15:486–490
Hung SC, Huang ML, Liu SM, Hsu HC (1999) Massive ascites caused by peritoneal extramedullary hematopoiesis as the initial manifestation of myelofibrosis. Am J Med Sci 318: 198–200
Jacobs P, Wood L, Robson S (1991) Refractory ascites in the chronic myeloproliferative syndrome: a case report. Am J Hematol 37:128–129
Jones RJ (1992) The role of bone marrow imaging. Radiology 183:321–322
Kaplan KR, Mitchell DG, Steiner RM, Murphy S, Vinitski S, Rao VM, Burk DL, Rifkin MD (1992) Polycythemia vera and myelofibrosis: correlation of MR imaging, clinical, and laboratory findings. Radiology 183:329–334
King BF, Kopecky KK, Baker MK, Clark SA (1987) Extramedullary hematopoiesis in the adrenal glands: CT characteristics. J Comput Assist Tomogr 11:342–343
Kobayashi A, Sugihara M, Kurosaki M, Ishida Y, Takayanagi N, Matsui O, Takashima T (1989) CT characteristics of intrahepatic, periportal, extramedullary hematopoiesis. J Comput Assist Tomogr 13:354–356
Koch BL, Bisset GS, 3rd, Bisset RR, Zimmer MB (1994) Intracranial extramedullary hematopoiesis: MR findings with pathologic correlation. AJR Am J Roentgenol 162:1419–1420
Kosmidis PA, Palacas CG, Axelrod AR (1980) Diffuse purely osteolytic lesions in myelofibrosis. Cancer 46:2263–2265
Kupferschmid JP, Shahian DM, Villanueva AG (1993) Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest 103:974–975
La Fianza A, Alberici E, Torretta L (2001) The irreplaceable image: Rapidly growing extramedullary hematopoiesis in lymph nodes: unusual findings of long-standing idiopathic myelofibrosis. Haematologica 86:784
Lanir A, Aghai E, Simon JS, Lee RG, Clouse ME (1986) MR imaging in myelofibrosis. J Comput Assist Tomogr 10: 634–636
Ligumski M, Polliack A, Benbassat J (1978) Myeloid metaplasia of the central nervous system in patients with myelofibrosis and agnogenic myeloid metaplasia. Report of 3 cases and review of the literature. Am J Med Sci 275:99–103
Lund RE, Aldridge NH (1984) Computed tomography of intracranial extramedullary hematopoiesis. J Comput Assist Tomogr 8:788–790
MacKinnon S, McNicol AM, Lee FD, McDonald GA (1986) Myelofibrosis complicated by intestinal extramedullary haemopoiesis and acute small bowel obstruction. J Clin Pathol 39:677–679
Mallouh AA, Sa’di AR (1992) Agnogenic myeloid metaplasia in children. Am J Dis Child 146:965–967
Navarro M, Crespo C, Perez L, Martinez C, Galant J, Gonzalez I (2000) Massive intrahepatic extramedullary hematopoiesis in myelofibrosis. Abdom Imaging 25:184–186
Oesterling JE, Keating JP, Leroy AJ, Earle JD, Farrow GM, McCarthy JT, Silverstein MN (1992) Idiopathic myelofi — brosis with myeloid metaplasia involving the renal pelves, ureters and bladder. J Urol 147:1360–1362
Ohtsubo M, Hayashi K, Fukushima T, Chiyoda S, Takahara O (1994) Case report: intracranial extramedullary haematopoiesis in postpolycythemic myelofibrosis. Br J Radiol 67:299–302
Ozsoylu S (1994) Myelofi brosis in children. Pediatr Hematol Oncol 11:339–340,347
Papavasiliou C, Gouliamos A, Deligiorgi E, Vlahos L, Cambouris T (1990) Masses of myeloadipose tissue: radiological and clinical considerations. Int J Radiat Oncol Biol Phys 19:985–993
Rapezzi D, Racchi O, Ferraris AM (1997) Perirenal extramedullary hematopoiesis in agnogenic myeloid metaplasia: MR imaging findings. AJR Am J Roentgenol 168:1388–1389
Redlin L, Francis RS, Orlando MM (1976) Renal abnormalities in agnogenic myeloid metaplasia. Radiology 121: 605–608
Rozman C, Cervantes F, Rozman M, Mercader JM, Montserrat E (1999) Magnetic resonance imaging in myelofi brosis and essential thrombocythaemia: contribution to differential diagnosis. Br J Haematol 104:574–580
Savader SJ, Otero RR, Savader BL (1988) MR imaging of intrathoracic extramedullary hematopoiesis. J Comput Assist Tomogr 12:878–880
Schnuelle P, Waldherr R, Lehmann KJ, Woenckhaus J, Back W, Niemir Z, van der Woude FJ (1999) Idiopathic myelofibrosis with extramedullary hematopoiesis in the kidneys. Clin Nephrol 52:256–262
Shaldon S, Sherlock S (1962) Portal hypertension in the myeloproliferative syndrome and the reticuloses. Am J Med 32: 758–768
Sharma BK, Pounder RE, Cruse JP, Knowles SM, Lewis AA (1986) Extramedullary haemopoiesis in the small bowel. Gut 27:873–875
Shaver RW, Clore FC (1981) Extramedullary hematopoiesis in myeloid metaplasia. AJR Am J Roentgenol 137:874–876
Siniluoto TM, Hyvarinen SA, Paivansalo MJ, Alavaikko MJ, Suramo IJ (1992) Abdominal ultrasonography in myelofi — brosis. Acta Radiol 33:343–346
Solomon D, Goodman H, Jacobs P (1994) Case report: rectal stenosis due to extramedullary haemopoiesis-radiological features. Clin Radiol 49:726–728
Ueda F, Takashima T, Suzuki M, Kadoya M (1994) MR diagnosis of myelofibrosis. Radiat Med 12:135–137
Urman M, O’Sullivan RA, Nugent RA, Lentle BC (1991) Intracranial extramedullary hematopoiesis. CT and bone marrow scan findings. Clin Nucl Med 16:431–434
Warshauer DM, Schiebler ML (1991) Intrahepatic extramedullary hematopoiesis: MR, CT, and sonographic appearance. J Comput Assist Tomogr 15:683–685
Wiener MD, Halvorsen RA, Jr., Vollmer RT, Foster WL, Roberts L, Jr. (1987) Focal intrahepatic extramedullary hematopoiesis mimicking neoplasm. AJR Am J Roentgenol 149:1171–1172
Wright RE (1991) Case report: pararenal extramedullary haematopoietic tissue-an unusual manifestation of myelofibrosis. Clin Radiol 44:210–211
Wyatt SH, Fishman EK (1994) Diffuse pulmonary extramedullary hematopoiesis in a patient with myelofibrosis: CT findings. J Comput Assist Tomogr 18:815–817
Yokoyama T, Saigo K, Maeda N, Hamada Y, Yano Y, Mori T, Tamura M, Tasaka K, Okutani T, Takata M, Maeda Y, Tomofuji Y, Chinzei T (2000) Tumor-like splenic extramedullary hematopoiesis in a patient with myelofibrosis. Intern Med 39:416–418
Yu JS, Greenway G, Resnick D (1994) Myelofi brosis associated with prominent periosteal bone apposition. Report of two cases. Clin Imaging 18:89–92
Yu TF (1965) Secondary gout associated with myeloproliferative diseases. Arthritis Rheum 8:765–771
Zonderland HM, Michiels JJ, ten Kate FJ (1991) Case report: mammographic and sonographic demonstration of extramedullary haematopoiesis of the breast. Clin Radiol 44:64–65
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2004 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Guermazi, A. (2004). Myelofibrosis. In: Guermazi, A. (eds) Radiological Imaging in Hematological Malignancies. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-18832-9_21
Download citation
DOI: https://doi.org/10.1007/978-3-642-18832-9_21
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-62313-4
Online ISBN: 978-3-642-18832-9
eBook Packages: Springer Book Archive